| Systemic mastocytosis in adults: 2011 update on diagnosis, risk stratification, and management. | |
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MedLine Citation:
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PMID: 21442641 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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DIAGNOSIS: The major criterion is presence of multifocal clusters of morphologically abnormal MC in the bone marrow. Minor diagnostic criteria include elevated serum tryptase level, abnormal MC expression of CD25 and/or CD2, and presence of KITD816V. RISK STRATIFICATION: The prognostic relevance of the 2008 World Health Organization (WHO) classification of SM has recently been confirmed. Classification of SM patients into indolent (SM), aggressive SM (ASM), SM associated with a clonal non-MC lineage disease (SM-AHNMD), and mast cell leukemia (MCL) subgroups is a useful first step in establishing prognosis. Risk-adapted therapy: SM treatment is generally palliative. ISM patients have a normal life expectancy and receive symptom-directed therapy; infrequently, cytoreductive therapy may be indicated for refractory symptoms. ASM patients have disease-related organ dysfunction; interferon-α (±corticosteroids) can control dermatological, hematological, gastrointestinal, skeletal, and mediator-release symptoms, but is hampered by poor tolerability. Similarly, cladribine has broad therapeutic activity, with particular utility when rapid MC debulking is indicated; the main toxicity is myelosuppression. Imatinib has a therapeutic role in the presence of an imatinib-sensitive KIT mutation or in KITD816-unmutated patients. Treatment of SM-AHNMD is governed primarily by the non-MC neoplasm; hydroxyurea has modest utility in this setting. Dasatinib's in vitro anti- KITD816V activity has not translated into significant therapeutic activity in most SM patients. In contrast, preliminary data suggest that Midostaurin may produce significant decreases in MC burden in some patients. |
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Authors:
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Animesh Pardanani |
Publication Detail:
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Type: Journal Article |
Journal Detail:
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Title: American journal of hematology Volume: 86 ISSN: 1096-8652 ISO Abbreviation: Am. J. Hematol. Publication Date: 2011 Apr |
Date Detail:
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Created Date: 2011-03-28 Completed Date: 2011-05-20 Revised Date: 2013-05-20 |
Medline Journal Info:
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Nlm Unique ID: 7610369 Medline TA: Am J Hematol Country: United States |
Other Details:
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Languages: eng Pagination: 362-71 Citation Subset: IM |
Copyright Information:
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Copyright © 2011 Wiley-Liss, Inc. |
Affiliation:
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Department of Medicine, Division of Hematology, Mayo Clinic, 200 First Street SW, Rochester, MN 55905, USA. pardanani.animesh@mayo.edu |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Adult Antineoplastic Agents / administration & dosage, therapeutic use* Bone Marrow / pathology Cladribine / administration & dosage, therapeutic use Disease-Free Survival Humans Hydroxyurea / administration & dosage, therapeutic use Interferon Type I / administration & dosage, therapeutic use Mast Cells / drug effects, metabolism, pathology Mastocytosis, Systemic* / diagnosis, drug therapy, etiology Piperazines / administration & dosage, therapeutic use Point Mutation Proto-Oncogene Proteins c-kit / genetics Pyrimidines / administration & dosage, therapeutic use Recombinant Proteins Risk Staurosporine / administration & dosage, analogs & derivatives, therapeutic use Thiazoles / administration & dosage, therapeutic use |
| Chemical | |
Reg. No./Substance:
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0/Antineoplastic Agents; 0/Interferon Type I; 0/PKC412; 0/Piperazines; 0/Pyrimidines; 0/Recombinant Proteins; 0/Thiazoles; 127-07-1/Hydroxyurea; 302962-49-8/dasatinib; 4291-63-8/Cladribine; 62996-74-1/Staurosporine; BKJ8M8G5HI/imatinib; EC 2.7.10.1/Proto-Oncogene Proteins c-kit |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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